Department of Endocrinology, Metabolism, Rheumatology and Nephrology Faculty of Medicine, Oita University, 1-1 Idaigaoka, Hsama-machi Yufu, Oita, 879-5593, Japan.
Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Oita, Japan.
CEN Case Rep. 2021 Feb;10(1):35-41. doi: 10.1007/s13730-020-00511-8. Epub 2020 Jul 26.
We report two cases of idiopathic multicentric Castleman disease (iMCD) with nephrotic syndrome (NS) treated with tocilizumab. Case 1 was a 58-year-old man diagnosed with iMCD prior to the onset of NS. Renal biopsy revealed membranous nephropathy, which was considered to be secondary membranous nephropathy associated with iMCD. Case 2 was a 49-year-old woman diagnosed with iMCD prior to NS. Renal biopsy revealed renal amyloidosis positive for Congo red staining and amyloid A protein immunostaining. In both the cases, the proteinuria improved after the initiation of glucocorticoid and tocilizumab therapy. Tocilizumab may be a good therapeutic choice for iMCD with NS.
我们报告了两例特发性多中心 Castleman 病（iMCD）伴肾病综合征（NS）的病例，均采用托珠单抗治疗。病例 1 为 58 岁男性，在 NS 发病前被诊断为 iMCD。肾活检显示膜性肾病，被认为是与 iMCD 相关的继发性膜性肾病。病例 2 为 49 岁女性，在 NS 发病前被诊断为 iMCD。肾活检显示刚果红染色阳性的肾淀粉样变性和淀粉样 A 蛋白免疫染色。在这两个病例中，蛋白尿在开始使用糖皮质激素和托珠单抗治疗后得到改善。托珠单抗可能是 iMCD 伴 NS 的一种较好的治疗选择。
